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Neurofibromatosis Type I 환자에서 부인두강에 발생한 거대 신경섬유종증 1례
이형석(Hyung Seok Lee),이승환(Seung Hwan Lee),허영돈(Young Don Huh),홍동균(Dong Kyun Hong),이윤서(Yoon Seo Lee) 대한두경부종양학회 2000 대한두경부 종양학회지 Vol.16 No.1
Neurofibromatosis type I or Von Recklinghausen's disease can occur at any site in the body. It is characterized by multiple spots on the skin-more than six spots greater than 1.5cm-, neurofibromas of the peripheral and centarl nervous system, and variety of other dysplastic abnormalities of the skin, bones, endocrine organs, nervous systems, and blood vessels. It is an autosomal dominant trait disease with a frequency of 1 of 3000. Neurofibromatosis is known to be complicated by malignancies. Neurofibromatosis is progressive disease and shows a marked variations in expression in affected individuals. In this report we describe a male patient with neurofibromatosis type I developed in the parapharyngeal space. The patient had huge mass at left parapharyngeal space and inguinal area. We successfully treated the patient with surgery without complication.